St. Pauls Researcher Investigates Cause of Common Interstitial Lung Disease



Above: Dr. Darryl Knight in his lab at St. Paul’s Hospital, where he is currently investigating idiopathic pulmonary fibrosis (IPF). He describes IPF as having lungs as scarred and stiff as crushed pop cans. Breathing with IPF is comparable to trying to inflate a crushed can.

Idiopathic pulmonary fibrosis (IPF) is a relentless disease characterized by scarring of the lung tissue between the air sacs and blood vessels. IPF affects over 200,000 people in North America alone, and 40,000 die each year. IPF has no cure, no effective treatment and has a mortality rate worse than heart failure and many cancers, with a median survival of only three years from diagnosis. Equally important is the poor quality of life, as patients with IPF suffer with persistent shortness of breath that feels like they are drowning. Dr. Darryl Knight, Canada Research Chair in Airway Disease; Michael Smith Foundation Career Investigator; Associate Director, UBC James Hogg Research Centre of the Institute for Heart + Lung Health at St. Paul’s Hospital is currently investigating the cells that produce a thick connective tissue that can build up inside the lungs and cause the scarring that impairs the ability to transfer oxygen to the bloodstream. These cells are called myofibroblasts. His research team, based out of St. Paul’s Hospital, has identified a protein that might regulate how myofibroblasts appear and function but do not die. Through the study, Dr. Knight hopes to determine how to reduce the activity of this protein, and therefore reduce the production of the connective tissue at the root of this disease.